FAQ Chiari Malformation

Chiari I Malformation

Can Chiari occur in those not born with the deformity?

Yes. This is called secondary Chiari and it can be caused by hydrocephalus, increase of intracranial pressure, tumors that displace the brain structures and trauma. Some people that have a shunt in the lower spinal canal may also develop secondary Chiari. The condition of tethered spinal cord can result in a Chiari malformation.

Can CM-I reoccur?

Some people have recurrent symptoms after a period of good response to surgical treatment. There are various causes for this and a careful evaluation is needed. Sometimes the cause is not the Chiari itself, but other conditions such as raised intracranial pressure, cervical discs causing headaches or occipital neuralgia (pain from the nerves at the back of the head).

Does CM-I cause disability?

Chiari I Malformation can cause temporary or permanent disabilities. Many troubling symptoms that prevented working and playing often disappear after surgery. Other symptoms may not be easily resolved and may take time and effort on the part of the patient and healthcare professionals to solve so that the patient may get back to a normal lifestyle.

I have CM-I, is there anything that I should not do?

Pay attention to how you respond to various activities and use this as a guide. For many people with Chiari, lifting weights, straining, bending forward or looking up may worsen their symptoms. Contact sports and activities such as riding high-speed roller coasters may aggravate symptoms.

If CM-I is present at the time of birth, why do symptoms show up so much later?

The reason that symptoms in many people do not show up until later in life is unknown. It may be related to the repeated brain pulsations and straining activities resulting in some increased crowding which slowly worsens over time. Many people report symptoms after an injury such as a motor vehicle accident, whiplash and falls.

The Chiari I Malformation presents in adults, children and occasionally infants. It occurs three times more often in women than in men. There is no particular ethnic or geographic distribution. The cause of the Chiari I Malformation is not known.

The CSF surrounds the brain and spinal cord and provides protection for the delicate nerves contained within and circulates constantly. Most of the symptoms of Chiari I Malformation can be attributed to obstruction of spinal fluid flow or compression of the lower brainstem and cranial nerves.

Children & Pregnancy

If you have CM-I and your child has neurological symptoms suggestive of Chiari malformation, ask your pediatrician about ordering an MRI scan of the brain for a positive diagnosis.

How can I help my family and friends understand what I am going through?

Sometimes it is difficult for family and friends to understand your disease because physical symptoms are usually invisible to others. The best remedy for understanding is education. Consider taking a family member with you when you have an appointment with your neurosurgeon. Have a short list of important questions (about three) that you would like to have answered during your visit.

Many of the signs and symptoms that occur in adults are also present in children; however, young children may not be able to accurately report their symptoms. Rarely, children may present with Cerebellar Fits or episodes of Paroxysmal Rage.

If I have CM-I will my children have it?

For most patients CM-I does not run in families. In some cases, however, it can be passed from the mother or father to their children.

If pregnant, do CM-I patients have to have a Cesarean section?

No. Many women have normal vaginal deliveries. This decision should be made after a discussion with your OB/GYN.

Diagnostic Studies

What does an MRI scan show?

A Magnetic Resonance Imaging (MRI) scan creates an anatomical picture of the brain and spinal cord. In Chiari it can show the degree of crowding at the large opening at the bottom of the skull called the foramen magnum. Brain tissue compression and distortion can be analyzed and the shape and changes in the bony structures can be evaluated. An MRI of the spine can show the presence of syringomyelia.

What is a CINE study and what is it used for?

A CINE study is a special MRI that is used to assess the flow of spinal fluid through the large opening at the base of the skull called the foramen magnum. It is used in some cases to determine if spinal fluid flow is being blocked.

Finding a Doctor

I have been diagnosed with CM-I, what type of doctor should I see?

You should see a neurosurgeon who is experienced in the evaluation and treatment of people with the Chiari I Malformation. A neurosurgeon is a specialized physician that evaluates and treats people with diseases and problems of the nervous system that may require surgery.

I take a variety of herbal supplements. Are there any that I should avoid with CM-I?

Herbal supplements do not have the scientific studies backing their effectiveness or safety that prescription drugs have. The pharmaceutical industry spends millions of dollars developing, testing, running clinical trials and getting FDA approval before a drug is released. The quality and strength of herbal preparations are not regulated in this country. The interactions of many herbal preparations with prescription drugs are not known. Prescription medications interactions with other prescriptions drugs and common foods or drinks are well understood. For example a common drug to lower cholesterol, Lipitor®, when taken with grapefruit juice can cause toxicity in some people.

Is it OK for my chiropractor to crack my neck?

High-velocity chiropractic is not advised in persons with Chiari I Malformation; injuries have occurred.

Hydrocephalus

I have CM-I and hydrocephalus. What is hydrocephalus?

Hydrocephalus is an accumulation of cerebral spinal fluid within the ventricles of the brain. It results from blockage of normal spinal fluid circulation. Hydrocephalus may result from developmental abnormalities, infection, brain injury and brain tumors.

What are the dangers of hydrocephalus?

The dilation of ventricles with spinal fluid places pressure on delicate brain tissues. Symptoms of hydrocephalus may include headache followed by vomiting, nausea, blurred vision, diplopia (double vision), problems with balance and coordination, difficulty walking, urinary incontinence, drowsiness, irritability and other changes in personality or memory changes.

Will CM-I surgery make the hydrocephalus better?

In some cases, hydrocephalus will resolve once the blockage of spinal fluid flow has been relieved by Chiari surgery. In others, a shunt may have to be placed in order to remove the excess fluid from the brain ventricles.

Sharing a Diagnosis

Should I tell my boss that I have CM-I?

Many people with the diagnosis of Chiari or syringomyelia continue to have productive careers long after they are diagnosed. There are many state and federal laws that address work issues for people with disabilities or chronic illness. Check with your state government about available hotlines and information about discrimination in the work place. There are two federal laws that may be useful: The Americans with Disabilities Act (ADA) and The Family and Medical Leave Act (FMLA).

After you know your rights, you may decide if you want your boss to know about your condition.

Symptoms

How can I manage my headaches?

Coughing, straining, laughing, bending or lifting often aggravates headaches. Headaches can also be caused by overuse of medications used to treat headaches. Persons suffering from headaches should make sure to drink an adequate amount of water as dehydration can contribute to headaches. Many people report some relief of headaches while resting in a darkened room, occasional use of migraine medication, application of heat or cold to the area and use of certain anti-seizure medications.

Is there anything I can do to help with dizziness?

Abrupt movements of the head and getting up quickly can cause dizziness. It is important to remember that there can be other causes of the symptoms commonly associated with Chiari. A consult with an ear, nose and throat specialist may help to rule out another reason for the dizziness, such as problems with the inner ear. Sudden movements or laying flat may increase dizziness.

The constant burning pain in my arms is keeping me from thinking well at work and sleeping. What can I take for this pain?

As with other types of neuropathic (nerve) pain, narcotic pain pills do not tend to work well. This type of pain is treated in a variety of ways. The most common treatment is with certain anti-seizure medications (gabapentin, pregabalin) that can be used to control neuropathic pain.

What other diseases can cause symptoms like CM-I?

There are many diseases that can cause some of the same symptoms as CM-I. This list does not include all disease states. A partial list of other causes are: hypo/hyperthyroid, vitamin deficiency, anemia, environmental toxins, alcoholism, medication toxicity or interactions, depression, anxiety or panic disorders, stroke, brain tumor (especially of the cerebellum), arachnoid cysts of the cerebellum, encephalopathy, cerebellar atrophy, multiple sclerosis, cerebellar abscess, brain trauma/injury, diabetic neuropathy, polyneuropathcy, post polio syndrome, disorders of the cervical or thoracic spine, syringomyelia, myasthenia gravis, amyotopic lateral sclerosis and neurosarcoidosis.

Why am I depressed and anxious? Should I be treated?

Depression and anxiety can be caused by chronic illness, pain and uncertainty. In some patients with Chiari, these symptoms may be due to the cerebellar cognitive affective syndrome and can improve following decompression surgery. Some patients are placed on medications for depression and anxiety.

Why am I falling and having trouble taking walks?

Chiari causes crowding of the cerebellar tonsils. The cerebellar portion of the brain is responsible for the coordination of voluntary muscular movements. The cerebellum interacts with the structures of the brainstem and helps to execute a variety of movements such as balance and walking.